Adrenal crisis in a 14-year-old boy 12 years after hematopoietic stem cell transplantation

Beitrag in einer Fachzeitschrift


Details zur Publikation

Autor(en): Penger T, Albrecht A, Marx M, Stachel D, Metzler M, Dörr HG
Zeitschrift: Endocrinology, Diabetes and Metabolism Case Reports
Jahr der Veröffentlichung: 2018
Band: 2018
ISSN: 2052-0573


Abstract

We report on a boy of Albanian descent with the history of juvenile myelomonocytic leukemia (JMML). JMML was diagnosed at the age of 17 months and treated by hematopoietic stem cell transplantation (HSCT). At the age of 14.3 years, about 12 years after HSCT, he was hospitalized with an adrenal crisis. Hormone findings were consistent with primary adrenal insufficiency. Autoimmune adrenalitis was confirmed by positive autoantibodies against 21-hydroxylase and adrenal tissue. Since autoimmune Hashimoto thyroiditis was already known from the age of 9 years, we assume that both diseases are part of the spectrum of autoimmune polyglandular syndrome (APS) type 2. APS type 2 is a rare endocrine disease characterized by Addison's disease along with autoimmune thyroid disease and/or type 1 diabetes.
Learning points: Endocrine sequelae after hematopoietic stem cell transplantation (HSCT) are common and can develop over a long period.Primary adrenal insufficiency after HSCT is absolutely rare.The combination of adrenal autoimmune disease and Hashimoto thyroiditis is consistent with autoimmune polyglandular syndrome type 2.


FAU-Autoren / FAU-Herausgeber

Albrecht, Andrea
Lehrstuhl für Kinderheilkunde
Dörr, Helmuth-Günther Prof. Dr.
Professur für Kinderheilkunde mit dem Schwerpunkt Kinder-Endokrinologie und Diabetologie (Stiftungsprofessur)
Metzler, Markus PD Dr.
Professur für Kinder- und Jugendmedizin mit dem Schwerpunkt Pädiatrische Onkologie und Hämatologie
Penger, Theresa
Professur für Kinderheilkunde mit dem Schwerpunkt Kinder-Endokrinologie und Diabetologie (Stiftungsprofessur)


Zitierweisen

APA:
Penger, T., Albrecht, A., Marx, M., Stachel, D., Metzler, M., & Dörr, H.-G. (2018). Adrenal crisis in a 14-year-old boy 12 years after hematopoietic stem cell transplantation. Endocrinology, Diabetes and Metabolism Case Reports, 2018. https://dx.doi.org/10.1530/EDM-18-0034

MLA:
Penger, Theresa, et al. "Adrenal crisis in a 14-year-old boy 12 years after hematopoietic stem cell transplantation." Endocrinology, Diabetes and Metabolism Case Reports 2018 (2018).

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Zuletzt aktualisiert 2019-25-03 um 10:53